Pulmonary Stenosis is a Predictor of Unfavorable Outcome After Surgery for Supravalvular Aortic Stenosis Pediatric Cardiology, 04/10/2012

Kasnar–Samprec J et al. – Pulmonary stenosis (PS) in patients with supravalvular aortic stenosis (SVAS) is a risk factor for reoperations in the aortic region and might be considered an indicator of the severity of the arterial disease and a predictor of an unfavourable outcome.

• The authors identified all patients with SVAS from the surgical database.
• The patients with multi–level aortic stenosis or concomitant cardiac procedures were excluded from this study.
• Follow–up (100 %) was conducted between 2008 and 2010.
• Twenty–six patients underwent surgery for SVAS between 1974 and 2006.
• Seventeen patients (65 %) were diagnosed with Williams–Beuren–Syndrome, six (17 %) had a diffuse form of SVAS and 10 (39 %) had PS.
• No patient had a surgical or interventional procedure for PS at the initial operation or during follow–up.

• There was no statistically significant association between PS and WBS (p=0.30) or diffuse form of SVAS (p=0.13).
• Patients with PS were operated at younger age (p=0.028).
• Median follow–up time was 14.6years.
• Overall mortality was 11.5 %.
• One patient with preoperatively severely decreased LV–function died 27days postoperatively.
• Two late deaths occurred 7 and 10years after the initial operation.
• Reoperations were required in 4 patients (15 %), 4–19years after the original operation, due to aortic arch stenosis, supravalvular restenosis or poststenotic aortic dilatation.
• PS was found to be a risk factor for reoperation (p=0.005) and for the combined reoperation/death end–point (p=0.003).